Marian Freedman

The North American Society for Pediatric and Adolescent Gynecology (NASPAG) recently released a clinical opinion on how to diagnose and manage vulvar lichen sclerosus (LS) based on a review of the literature. This clinical opinion replaces NASPAG’s 2014 recommendations.

Vulvar LS, a chronic inflammatory skin condition often affecting prepubertal girls, is characterized by vulvar irritation and pain, vulvar pruritus, bleeding due to skin fissures, painful defecation, constipation, and sometimes urinary tract symptoms. LS usually appears as ivory white or rose-colored patches, which can spread to the perineal and perianal regions to create a “figure 8” shape. Genital scarring may develop, resulting in labial and clitoral hood adhesions, a buried clitoris, loss or attenuation of labia minora, and narrowing of the vaginal opening.

LS generally can be diagnosed based on the clinical exam, followed by a therapy trial. If the treatment fails or the diagnosis is in doubt, a referral to a pediatric dermatologist or pediatric gynecologist may be advisable.

Treatment mainstays include symptom management and ultrahigh potency topical corticosteroids (UPTC), generally clobetasol propionate and augmented betamethasone dipropionate, used daily or until symptoms are controlled. Ointments are preferable to creams because of their increased penetration and decreased risk of contact dermatitis. Topical calcineurin inhibitors also have been used to treat LS.

Many patients continue to have mild itching and discomfort even after appropriate treatment. Good vulvar hygiene and cool compresses can ease these symptoms.

Because LS often relapses, NASPAG recommends maintenance treatment with a daily topical calcineurin inhibitor or with tapered topical steroids. If LS hasn’t progressed after 6 months of maintenance therapy, the patient should be checked yearly for another 2 years before considering discontinuing therapy if symptoms are well controlled.

The clinical opinion also noted that although UPTCs can resolve symptoms of pruritus and pain, long-term anatomical distortions can persist, leading to negative effects on sexual function and other aspects of quality of life. Clinicians, therefore, should counsel adolescents with LS to have regular gynecologic follow-up.

This condition is not that unusual. I’ve seen it several times, and the diagnosis is straightforward if you’ve seen it (or pictures of it) before. It is not due to sexual abuse, and unlike with most rashes in the area, antifungal creams or low-potency topical steroids are not the treatment of choice.

1. Simms-Cendan J, Hoover K, Marathe K, Tyler K. NASPAG clinical opinion: diagnosis and management of lichen sclerosus in the pediatric and adolescent patient. 

Published online October 2, 2021. doi:10.1016/j.jpag.2021.09.008

Articles

The North American Society for Pediatric and Adolescent Gynecology (NASPAG) has provided guidance for diagnosing and managing vulvar lichen sclerosus in pediatric patients.

NASPAG provides clinical opinion on vulvar lichen sclerosus

A retrospective review from Israel based on analysis of the medical charts of 32 healthy children diagnosed with prolonged, isolated aspartate aminotransferase (AST) elevation at a mean age of 39 months found that none developed significant liver disease. This was true even though at follow-up (at a mean of 65.6 months), AST generally remained elevated—from 1.23 to 12 times the up- per limit of normal—with the abnormality resolving in only 10 patients.

Levels of macro-AST (m-AST) were relatively prevalent; 22% of the children were m-AST positive, and 44% had borderline levels. However, AST levels were no higher in those who were m-AST positive than in those characterized as borderline or negative for m-AST. Investigators did find that the age at presentation was significantly different in those with borderline m-AST levels and those with m-AST–positive levels: 31 months versus 69 months, respectively.

Given these findings, investigators determined that in a healthy child with prolonged, isolated AST elevation and normal lactate dehydrogenase, alanine transaminase, and creatine kinase, further laboratory and imaging investigation usually is unnecessary.

We have all often seen this in healthy children and should learn to ignore it, unless clinical findings suggest liver disease or the child is on medicine that is potentially toxic to the liver. Nonetheless, unlike the authors, I would probably still feel compelled to repeat testing in a child with a level 12 times above normal.

1. Magen-Rimon R, Tal G, Kaplan M, Shaoul R. The significance of isolated aspartate aminotransferase elevation in healthy paediatric patients. 

Published online September 26, 2021. doi:10.1111/apa.16123

A study examines where prolonged, elevated aspartate aminotransferase (AST) when it's in isolation is indicative of liver disease or not a significant occurrence. 

Prolonged, isolated AST elevation is likely benign

A survey of surgeons who perform posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) found that when practitioners allow these patients to return to sports depends on physician-related factors. Conversely, patient-related factors did not impact protocols.

Investigators gave the 170 survey participants a questionnaire that presented clinical vignettes involving patients with AIS who had undergone PSF and differed in various ways, such as sex, age, obesity, skeletal maturity, levels fused, and extension of the fusion construct into lumbar vertebrae. Surgeons were asked to list when they would allow patients to return to 4 categories of activity: jogging, non-contact, contact, and collision sports.

Overall, surgeons recommended taking more time to return to more physically demanding sports (collision, 9.8 months; contact, 6.8 months; noncontact, 4.6 months; jogging, 4.1 months), and surgeons with more years in practice and higher case volume allowed faster return to activity.

Surgeon volume was a major factor in decisions; for example, surgeons who performed 25 to 50 PSFs annually allowed the quickest return to jogging and noncontact sports. Similarly, surgeons with 16 to 20 years of experience allowed a faster return to jogging than their peers with less experience.

Pediatric orthopedic–trained surgeons called for a faster return to sports than their colleagues who had other kinds of fellowships. Finally, surgeons working in community children’s hospitals allowed more rapid return to sports activity than their colleagues in academic and community general/adult settings.

Bottom line, there is no good science to back up recommendations, so we are flying blind here. If a child is being restricted for what seems to you an unduly long time, you may want to encourage the orthopedist to rethink his or her position (eg, by having him or her talk to colleagues).

1. Ho D, Du JY, Erkilinc M, Glotzbecker MP, Mistovich RJ. Getting them back in the game: when can athletes with adolescent idiopathic scoliosis safely return to sports? a mixed-effects study of the Pediatric Orthopaedic Association of North America. 

. 2021;41(9):e717-e721. doi:10.1097/BPO.0000000000001902

What's the proper timing for return to sports after posterior spinal fusion to treat adolescent idiopathic scoliosis (AIS)? A recent study offers insight into how surgeon volume and experience may impact patient recommendations.

When can athletes return to sports after AIS surgery?

Measurements of hemoglobin (Hb) using a noninvasive monitor positively correlate with measurements based on a traditional blood draw, a study conducted in Tokyo, Japan revealed. The 120 participants, aged 9 to 15 years, first had their Hb levels measured using the portable Astrim Fit device, which estimates levels using near-infrared spectroscopy combined with optical imaging. After undergoing the noninvasive procedure, the children had blood drawn, and investigators analyzed and compared the 2 sets of data. The comparison revealed an extremely low bias, no systematic bias relative to both fixed and proportion biases, and a positive correlation in Hb levels between those obtained with the noninvasive monitoring device and those from blood collection. Although the investigators said that agreement between the 2 methods needs to be improved before noninvasive monitoring can be used for medical diagnosis, they concluded that the device may be useful for evaluating Hb levels among schoolchildren in a health promotion or research setting.

Modern technology continues to amaze. This is not ready for public use yet and will also need to be improved so we can use it on infants but will be another breakthrough when it comes. And, as with sublingual allergy therapy, will further expand our ability to treat children noninvasively.

1. Okazaki K, Okazaki K, Uesugi M, Matsusima T, Hataya H. Evaluation of the accuracy of a non-invasive hemoglobin-monitoring device in schoolchildren. Pediatr Neonatol. Published online July 24, 2021. doi: 10.1016/j.pedneo.2021.05.028

A new noninvasive technology could offer an alternate and accurate way to measure hemoglobin (Hb) levels.

Noninvasive device accurately monitors Hb levels

Peanut oral immunotherapy (OIT) can benefit children of all ages, even adolescents and those with markedly elevated baseline peanut-specific immunoglobulin E (sIgE), according to new study findings. Investigators reviewed the records of children undergoing peanut OIT at a pediatric allergy clinic, then analyzed how patient age, peanut skin prick test results, and peanut sIgE results correlated with outcomes.

Patients took small peanut doses daily with other food. If they tolerated the initial dose well, the dose was double after 2 weeks. Long-term maintenance dosing was 2 teaspoons of peanut butter or 10 whole peanuts or 1 teaspoon of peanut butter or 5 peanuts for toddlers. Patients on daily maintenance dosing returned to the clinic every 6 months and were permitted unrestricted peanut intake if they tolerated at least 7000 mg of peanut protein.

Of 174 children receiving OIT, 144 achieved maintenance dosing, and 50 of those children transitioned to twice-weekly ad lib peanut ingestion. Thirty children discontinued OIT, most often during updosing and generally because of aversion to peanut or gastrointestinal symptoms. Of 47 patients who underwent multifood OIT with additional foods, 40 were updosed for these extra foods simultaneously with peanut and the other 7 after reaching their peanut maintenance dose. These patients did not differ from those on peanut OIT alone in reactions or time to reach maintenance. Although about two-thirds of patients experienced at least 1 adverse reaction during OIT, most of them—generally abdominal pain or nausea—were mild and responded to treatment with H

Out was most likely to be successful in children in whom it was started at a young age, with 92% of patients up to 5 achieving maintenance. This compares with 51% of those who were aged 5 to 11 years when they started OIT and 70% of those aged 1 to 18 years. In addition, baseline peanut sIgE level positively correlated with the number of reactions patients experienced.

This was a real-life study not done as part of a formal research protocol. As with preventing allergies by introducing peanuts at an early age, starting therapy early is better and, of course, with sublingual treatment, much easier on the child. Let’s hope your local allergists (or you yourself, if so inclined) get on board with this soon.

1. Guarnieri KM, Slack IF, Gadoury-Levesque V, Eapen AA, Andorf S, Lierl MB. Peanut oral immunotherapy in pediatric allergy clinic: patient factors associated with clinical outcomes. 

. 2021;127(2):214-222. doi:10.1016/j.anai.2021.04.003

Peanut oral immunotherapy has been beneficial to children with peanut allergies. A new study looks at just how effective it is.

How successful is peanut immunotherapy?

A milestone-related assessment of early psychomotor development of preterm (PT) twins compared with PT singletons found that twins born between 32 and 33 weeks’ gestational age (GA) have an early neuro- development advantage over their singleton peers. Italian investigators conducted developmental assessments in 73 PT twins and 207 PT singletons throughout the first 18 months of life. Assessments, using standardized tests and parental observation, included primary gross motor milestones, beginning of babbling, early visual fixation, and being able to follow a moving object. Investigators divided participants into 3 groups according to GA: 17 born at less than 31 weeks (group 1), 30 born between 32 and 33 weeks (group 2), and 26 born between 34 and 36 weeks (group 3). Twins in group 1 not only spoke their first single word significantly earlier than singletons in the group did but also demonstrated persistent superior language skill achievement at long-term follow-up together with better oculomanual abilities. Compared with singletons, group 2 twins also spoke their first single words at a younger age and achieved standing, walking, and pointing earlier. No significant differences emerged between group 3 twins and singletons except that twins demonstrated worse personal and social skills than singletons at long-term follow-up. Overall, investigators concluded that the differences in twins compared with singletons in the 3 groups were independently related to their gemellarity (“twinness”).

This is an interesting finding. An earlier study showed that firstborn twins above 28 weeks’ gestation were less likely to have respiratory distress syndrome than singletons. I wonder whether better lungs account for some of the difference. Unfortunately, many of the children, twins or not, had developmental delays at 25 to 36 months of corrected age.

1. Turriziani L, Cafeo A, Pino G, Vetrano N, Alibrandi A, Di Rosa G. Early psychomotor advantage in moderately preterm twins born between 32 and 33 weeks. 

 2021;19(4):240-246. doi:10.1055/s-0040-1709719

A recent study indicates that when born prematurely, twins may have some gains over singleton babies.